{"product_id":"fabry-disease-by-deborah-elstein","title":"Fabry Disease by Deborah Elstein | ISBN-13 9789400798847","description":"\u003cp\u003e\u003cb\u003eFabry Disease by Deborah Elstein\u003c\/b\u003e\u003c\/p\u003e\n Fabry disease is an X-linked inborn error of metabolism wherein deficiency of a lysosomal enzyme results in systemic deposition of glycosphingolipids. Storage deposition, and hence pathological disease, occurs preferentially in renal glomerular and tubular epithelial cells, myocardial cells, heart valve fibrocytes, neurons of dorsal root ganglia, and in endothelial smooth muscle cells of blood vessels. Thus, Fabry disease is a multi-system disorder, albeit with considerable phenotypic heterogeneity in onset and in severity; however, it is progressive, exhibits extensive morbidity, and is life-threatening. Within the past two decades, there has been a radical change in the natural course Fabry disease by virtue of the availability of specific enzyme replacement therapy. Moreover, there has been a concerted effort to better understand the underlying pathology and equally to identify patients prior to the onset of irreversible end-organ damage. It is to be hoped that the future for patients with Fabry disease can be viewed with greater, albeit guarded, optimism. This state-of-the-art textbook attempts to bridge the span of pre-clinical studies, clinical finding, and management options in a readable but comprehensive manner for the medical practitioner as well as the interested non-medical reader.\u003ch3\u003eBook details\u003c\/h3\u003e\u003ctable style=\"border-collapse:collapse;width:100%;\"\u003e\u003ctbody\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eISBN 13\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003e9789400798847\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eISBN 10\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003e9400798849\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eAuthor\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003eDeborah Elstein\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eCondition\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003eNew\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eBinding Type\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003ePaperback\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003ePublisher\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003eSpringer\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eYear published\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003e2014-09-29\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eNumber of pages\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003e512\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003cth style=\"text-align:left;padding:6px 10px;border:1px solid #ddd;\"\u003eCover note\u003c\/th\u003e\n\u003ctd style=\"padding:6px 10px;border:1px solid #ddd;\"\u003eBook picture is for illustrative purposes only, actual binding, cover or edition may vary.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\u003c\/table\u003e","brand":"Springer","offers":[{"title":"Default Title","offer_id":67682274509045,"sku":"9789400798847","price":39.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0795\/8632\/8821\/files\/9789400798847.jpg?v=1791826145","url":"https:\/\/arbiyas.com\/products\/fabry-disease-by-deborah-elstein","provider":"Arbiyas","version":"1.0","type":"link"}